Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a progressive liver disease that can eventually cause liver failure over a span of 15 to 20 years. After receiving a PBC diagnosis, you’ll want to know how this condition may affect your life expectancy, or how long you’re expected to live.

Your doctor may be able to predict your prognosis (outcome) by looking at several factors, including:

• Your PBC stage
• The results of your liver function tests
• Your symptoms
• Your PBC treatment

Continue reading to learn more about how each of these factors can help predict your life expectancy and prognosis with PBC.

How Does PBC Stage Affect Life Expectancy and Prognosis?

PBC worsens gradually, progressing through four different stages. Your doctor can tell which stage you’re in by taking a sample of liver tissue with a liver biopsy. Although a liver biopsy isn’t necessary to diagnose PBC, it can help predict your prognosis and how well you may respond to therapy.

There are four stages of PBC. Stages 1 and 2 are early stages of PBC that are characterized by inflammation and fibrosis (tissue stiffening caused by scarring) in the area around the portal vein. Stage 3 is characterized by changes in the structure of the liver caused by scarring. Stage 4 is characterized by cirrhosis — severe liver scarring that can lead to liver failure. Doctors may use your PBC stage, in addition to other factors of your PBC and overall health, when predicting your outcome and deciding on your treatment plan.

How Can Liver Function Tests Predict Life Expectancy and Prognosis?

Liver function tests can check the health of your liver by measuring the levels of substances in your blood made by the liver. Your doctor may use the results of this test to predict the course of your disease and how you may respond to treatment.

Bilirubin

Researchers have found that bilirubin levels are one of the best predictors of survival for people with PBC. Your liver makes bilirubin (a waste product) when it breaks down red blood cells (RBCs). Bilirubin levels are measured in milligrams per deciliter (mg/dL). Normal bilirubin levels in adults range from 0.2 mg/dL to 1.3 mg/dL.

Bilirubin levels may help predict how much longer you live. According to research, the average survival time (or time a person lives after diagnosis) based on bilirubin level is as follows:

• Bilirubin constantly above 2 mg/dL — Four years
• Bilirubin constantly above 6 mg/dL — Two years
• Bilirubin constantly above 10 mg/dL — 1.4 years

However, these are just averages and shouldn’t be the only factor used to predict outcomes with your specific case of PBC.

Alkaline Phosphatase

Alkaline phosphatase (ALP) is an enzyme (a type of protein) that’s primarily found in your liver. High levels of ALP can indicate liver problems.

Your ALP level can help predict how you will respond to treatment. People with ALP levels less than twice the upper limit of normal levels have a higher chance of responding to treatment with ursodiol — also called ursodeoxycholic acid (UDCA) — the first treatment option for people with PBC.

How Can PBC Symptoms Affect Life Expectancy?

PBC symptoms (or lack of symptoms) can help predict your life expectancy and prognosis. In general, more severe symptoms in the early stages of PBC may predict a faster disease progression.

People who are asymptomatic (do not have PBC symptoms) are often in an early stage of the disease. Between 50 percent and 70 percent of people with asymptomatic PBC are expected to live at least 10 years without treatment. About 1 in 4 people with asymptomatic PBC will progress to liver failure within 10 years.

People who are symptomatic (have PBC symptoms) are usually in a later stage of PBC. Without treatment, half of people with symptomatic PBC are expected to live between five and eight years.

Researchers haven’t found which factors increase your likelihood of developing PBC symptoms. People with PBC who never develop symptoms will likely have the same life expectancy as that of the general population.

What Are the Symptoms of PBC?

The most common early symptoms of PBC are fatigue and pruritus (itching). Other possible PBC symptoms include:

• Dry eyes and dry mouth
• Stomach pain
• Muscle and joint pain
• Changes in skin pigmentation
• Jaundice (yellowing of the skin and whites of the eyes)
• Xanthomas (fatty deposits under your skin)
• Osteoporosis (a condition that causes weak bones)
• High cholesterol
• Hypothyroidism (a condition that causes low thyroid hormone)

Fatigue is often considered the most debilitating symptom of PBC and can significantly decrease your quality of life. However, more severe fatigue and itching aren’t associated with more severe PBC. Researchers in a 2010 study of people with PBC suggested that high levels of fatigue may predict a higher likelihood of liver-related death and the need for a transplant.

How Can PBC Treatment Affect Life Expectancy and Prognosis?

With appropriate treatment, the life expectancy for people with PBC can be the same as that of the general population.

Ursodiol

Ursodiol (Actigall or Urso) is the first treatment used for people with PBC. It’s commonly referred to as UDCA (which, as noted, stands for ”ursodeoxycholic acid”).

UDCA can help to slow the progression of PBC to cirrhosis and help decrease the need for a liver transplant. UDCA is more effective at preventing the progression of PBC in people with stage 1 or stage 2 PBC.

People with a good response to UDCA in the first six months to two years usually have a better prognosis. Between 25 percent and 30 percent of people with PBC taking UDCA have a complete response to this treatment, according to an article in the journal Hepatic Medicine: Evidence and Research. A complete response means that PBC doesn’t progress to the next stage, and all liver function test results are normal.

People with a good response to UDCA can live as long as people without PBC. A 2005 study examined the survival rate of more than 250 people with PBC treated with UDCA. Researchers found that those treated with UDCA in the early stages of PBC had a similar survival rate as the general population.

Elafibranor

Elafibranor (Iqirvo) is a new PBC treatment approved by the U.S. Food and Drug Administration (FDA) in June 2024. It may be taken alone or in combination with UDCA.

In clinical trials involving people with PBC, elafibranor was shown to reduce levels of alkaline phosphatase (ALP), which are associated with liver function. It’s not yet confirmed if the drug improves survival in people with PBC, but researchers are further testing the drug in clinical trials.

Obeticholic Acid

Obeticholic acid (Ocaliva) is another treatment for PBC that was approved by the FDA in 2016.

While obeticholic acid can improve liver function and slow fibrosis, it doesn’t appear to improve survival. However, this medication is relatively new, so more clinical trials are needed to see the long-term effect of this medication in people with PBC.

Liver Transplant

If other treatment options don’t stop the progression of PBC, you may need a liver transplant. Liver transplantation is the only effective treatment for people with liver failure due to PBC. A liver transplant involves surgery to replace your diseased liver with a healthy liver donated by another person.

The article in Hepatic Medicine states that about 85 percent of people who receive a liver transplant live at least five more years. However, researcher have found that PBC comes back in about 20 percent of people three to seven years after they receive a new liver.

Prognostic Models for PBC

Your gastroenterologist (a doctor who specializes in treating conditions of the digestive system) may use a prognostic model to predict how well you’ll respond to treatment, your risk of needing a liver transplant, or your risk of liver-related death. Currently, there are two predictive models available — the GLOBE score and the UK-PCB score. Prognostic models are a tool to help predict your prognosis using specific information, such as your:

• Age
• Bilirubin level
• Albumin level
• ALP level
• Platelet level

Talking with your gastroenterologist about prognostic models for PBC, like the GLOBE score and the UK-PCB score, is important to understand your prognosis. These tools help figure out risks and make medical decisions easier. Don’t hesitate to ask questions or seek clarification during these talks. Your gastroenterologist is there to not only diagnose and treat but also guide you through your condition. This teamwork between you and your health care provider contributes to your overall well-being in managing PBC.

Talk With Others Who Understand

On myPBCteam, the social network for people with PBC and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with PBC.

How did your health care team discuss your PBC prognosis with you? What steps are you taking to improve your life expectancy and quality of life with PBC? Share your experience in the comments below, or start a conversation by posting on your Activities page.